{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,2]],"date-time":"2026-01-02T07:38:31Z","timestamp":1767339511300,"version":"3.41.2"},"reference-count":11,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2020,6,23]],"date-time":"2020-06-23T00:00:00Z","timestamp":1592870400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2020,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Deficiency of the CD40L, expressed on the surface of T lymphocytes, is caused by mutations in the glycoprotein <jats:italic>CD40L (CD154)<\/jats:italic> gene. Resulting defective humoral and cellular responses cause a clinical presentation that includes recurrent sinopulmonary bacterial infections, opportunistic infections, sclerosing cholangitis, neutropenia, and autoimmune manifestations. HSCT represents the only curative treatment modality. However, the therapeutic decision to use HSCT proves challenging in many cases, mainly due to the lack of a phenotype\u2010genotype correlation. We retrospectively reviewed\u00a0patients with CD40L deficiency who were transplanted in Antalya and G\u00f6ztepe MedicalPark Pediatric HSCT units from 2014 to 2019 and followed by Akdeniz University School of Medicine Department of Pediatric Immunology. The records of eight male cases, including one set of twins, were evaluated retrospectively. As two transplants each were performed on the twins, a total of ten transplants were evaluated. Conditioning regimens were predominantly based on myeloablative protocols, except for the twins, who received a non\u2010myeloablative regimen for their first transplantation. Median neutrophil and platelet engraftment days were 13 (range 10\u201019) and 14 (range 10\u201042) days, respectively. In seven of ten transplants, a CMV reactivation was developed without morbidity. None of the patients developed GVHD, except for one mild case of acute GVHD. All patients survived, and the median follow\u2010up was 852\u00a0days. Our data show that HSCT for patients with CD40 ligand deficiency is a potentially effective treatment for long\u2010term disease control.<\/jats:p>","DOI":"10.1111\/petr.13768","type":"journal-article","created":{"date-parts":[[2020,6,23]],"date-time":"2020-06-23T14:47:07Z","timestamp":1592923627000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Hematopoietic stem cell transplantation in CD40 ligand deficiency: A single\u2010center experience"],"prefix":"10.1111","volume":"24","author":[{"given":"Dilara Fatma Kocac\u0131k","family":"Uygun","sequence":"first","affiliation":[{"name":"Department of Pediatric Allergy and Immunology Akdeniz University School of Medicine Antalya Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3257-7798","authenticated-orcid":false,"given":"Vedat","family":"Uygun","sequence":"additional","affiliation":[{"name":"Department of Pediatric Bone Marrow Transplantation Unit MedicalPark Antalya Hospital Istinye University Antalya Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G\u00fcls\u00fcn Tezcan","family":"Karasu","sequence":"additional","affiliation":[{"name":"Department of Pediatric Bone Marrow Transplantation Unit MedicalPark G\u00f6ztepe Hospital \u0130stinye University School of Medicine \u0130stanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hayriye","family":"Dalo\u011flu","sequence":"additional","affiliation":[{"name":"Department of Pediatric Bone Marrow Transplantation Unit MedicalPark Antalya Hospital Antalya Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seda Irmak","family":"\u00d6zt\u00fcrkmen","sequence":"additional","affiliation":[{"name":"Department of Pediatric Bone Marrow Transplantation Unit MedicalPark Antalya Hospital Antalya Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fatih","family":"\u00c7elmeli","sequence":"additional","affiliation":[{"name":"Department of Allergy and Immunology University of Health Sciences Antalya Training and Research Hospital Antalya Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Selda Han\u00e7erli","family":"T\u00f6r\u00fcn","sequence":"additional","affiliation":[{"name":"Department of Pediatric Infection \u0130stanbul Faculty of Medicine \u0130stanbul University \u0130stanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ahmet","family":"\u00d6zen","sequence":"additional","affiliation":[{"name":"Pendik Department of Pediatric Allergy and Immunology School of Medicine Marmara University \u0130stanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Safa","family":"Bar\u0131\u015f","sequence":"additional","affiliation":[{"name":"Pendik Department of Pediatric Allergy and Immunology School of Medicine Marmara University \u0130stanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elif Karako\u00e7","family":"Ayd\u0131ner","sequence":"additional","affiliation":[{"name":"Pendik Department of Pediatric Allergy and Immunology School of Medicine Marmara University \u0130stanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Koray","family":"Yal\u00e7\u0131n","sequence":"additional","affiliation":[{"name":"Department of Pediatric Bone Marrow Transplantation Unit MedicalPark G\u00f6ztepe Hospital \u0130stanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Suar \u00c7ak\u0131","family":"K\u0131l\u0131\u00e7","sequence":"additional","affiliation":[{"name":"Department of Pediatric Bone Marrow Transplantation Unit MedicalPark G\u00f6ztepe Hospital \u0130stanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Volkan","family":"Hazar","sequence":"additional","affiliation":[{"name":"Department of Pediatric Bone Marrow Transplantation Unit MedicalPark G\u00f6ztepe Hospital \u0130stanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ay\u015fen","family":"Bing\u00f6l","sequence":"additional","affiliation":[{"name":"Department of Pediatric Allergy and Immunology Akdeniz University School of Medicine Antalya Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akif","family":"Ye\u015filipek","sequence":"additional","affiliation":[{"name":"Department of Pediatric Bone Marrow Transplantation Unit MedicalPark Antalya Hospital Antalya 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